Disruption of the Autophagy-Lysosome Pathway Is Involved in Neuropathology of the nclf Mouse Model of Neuronal Ceroid Lipofuscinosis

Variant late-infantile neuronal ceroid lipofuscinosis, a fatal lysosomal storage disorder accompanied by regional atrophy and pronounced neuron loss in the brain, is caused by mutations in the CLN6 gene. CLN6 is a non-glycosylated endoplasmic reticulum (ER)-resident membrane protein of unknown funct...

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Detalles Bibliográficos
Autores principales: Thelen, Melanie, Daμμe, Markus, Schweizer, Michaela, Hagel, Christian, Wong, Andrew M.S., Cooper, Jonathan D., Braulke, Thomas, Galliciotti, Giovanna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2012
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3335005/
https://www.ncbi.nlm.nih.gov/pubmed/22536393
http://dx.doi.org/10.1371/journal.pone.0035493

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3335005/
https://www.ncbi.nlm.nih.gov/pubmed/22536393
http://dx.doi.org/10.1371/journal.pone.0035493

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